Summary:Hematopoietic stem cell transplantation (HSCT) is a treatment option for autoimmune diseases but can also cause clinical features similar to those of autoimmune diseases. In some of these cases the autoimmune-like condition is associated with autoimmune cytopenia, a complication that can be unresponsive to established treatment strategies and which may be fatal. The majority of cases reported on immune hemolytic anemia have been of alloimmune origin due to ABO red blood cell antigen incompatibilities between donor and recipient. We now report a patient with a lupus-like syndrome, presenting with severe thrombocytopenia and hemolytic anemia 9 months after HLA-mismatch, ABO compatible-related PBSCT who experienced no response to high-dose steroids, but who had a sustained response to repeated IvIG therapy. Bone Marrow Transplantation (2001) 27, 337-340. Keywords: autoimmune cytopenia; hematopoietic stem cell transplantation; lupus-like syndrome; alloimmunity; IvIG Hematopoietic stem cell transplantation (HSCT) can cause clinical symptoms characteristic of autoimmune diseases like myasthenia gravis, immune thyroiditis, as well as immune cytopenia. 1-11 Especially, autoimmune cytopenia is mostly refractory to standard therapeutic approaches. 2,3 We herein report a patient with a lupus-like syndrome associated with hemolytic anemia and severe thrombocytopenia after HSCT.
Patient characteristicsNine months (day +277) after a mismatched related allogeneic stem cell transplant (PBSCT) for CML in first CP, a 38-year-old patient presented with weight gain, general GvHD prophylaxis consisted of cyclosporine (CYA) starting on day −3, prednisolone starting on day +7 after transplantation and MTX given as a short course on days +1, +3 and +6 at a dose of 15 mg/m 2 and twice at 10 mg/m 2 , respectively. Engraftment (leukocyte count Ͼ1000 × 10 9 /l, neutrophil count Ͼ0.5 × 10 9 /l) occurred on day +21 after PBSCT. He developed slight cutaneous GVHD grade I-II which responded promptly to increased immunosuppression with prednisolone (0.7 mg/kg bw × day) for 1 week, then tapered, and mycophenolate-mofetil (MMF) (2 g/day) for 3 months.CMV reactivation, as diagnosed by CMV-PCR, occurred 2 months after PBSCT (recipient CMV IgG positive, donor CMV IgG negative). This was successfully treated with ganciclovir, but the PCR became positive again shortly after cessation of therapy. He therefore received cidofovir as maintenance for 2 months.Immunosuppressive therapy on admission consisted of prednisolone 5 mg/day and CYA 150 mg/day given orally (blood level 284 ng/ml).
Clinical findingsAs papular cutaneous lesions were the main clinical finding a viral infection was suspected, possibly herpes virus or VZV reactivation. CMV testing had been negative for the last 4 months. However, the CMV-PCR was positive at the time of admission. He was treated with foscarnet to avoid further myelosuppression in a graft which was already suboptimal. Testing for VZV and HSV was negative.Within a few days of admission the patient developed
We studied the role of interleukin (IL)-8 during engraftment after hematopoietic stem cell transplantation. In 40 consecutive patients undergoing either allogeneic marrow (n=32) or autologous peripheral blood stem cell transplantation (n=8), IL-8 plasma levels were serially determined. Median IL-8 concentrations peaked during the neutropenic phase and, subsequently, subsided to pretransplant levels in patients achieving engraftment. In all patients, we observed an inverse correlation of IL-8 with leukocytes (P<0.0001) and a direct correlation of IL-8 with the extent of neutropenia (P<0.0001). Four patients who developed graft failure showed sustained median IL-8 concentrations of >300 pg/mL together with persistent neutropenia. This marked elevation of IL-8 was statistically significant as early as day 11 after transplantation, at a time when no other evidence alluded to imminent graft failure. Our data suggest that IL-8 may play an important role during engraftment after hematopoietic stem cell transplantation.
Granulocyte colony-stimulating factor-mediated acceleration of neutrophil recovery following myeloablative therapy correlated with increased IL-1ra plasma concentrations. Our data suggest that IL-1ra constitutes an intrinsic component of the anti-inflammatory and neutrophil differentiating efficacy of G-CSF and, thus, IL-1ra may be required for the in vivo activity of G-CSF.
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